[因子X缺乏与全身性淀粉样变性]。

F J Pedinielli, B Mascret, A P Blanc, N Sudan, I Juhan-Vague, Y Carcassonne
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引用次数: 0

摘要

报告一例52岁患者,经骨髓活检标本检查诊断为全身性淀粉样变性。最突出的临床表现是大出血综合征,这被认为是严重斯图尔特因子缺乏的结果。回顾了基于最新生化和免疫学发现的直链淀粉的新分类。目前的生理病理推测强调浆细胞-巨噬细胞的合作。最后,斯图尔特因子缺乏症既常见又罕见,它可能源于淀粉样纤维对该因子的特殊亲和力和“质量效应”(直接暴露于血流的淀粉样物质的数量),这解释了为什么脾切除术在某些情况下是有效的。
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[Factor X deficiency and systemic amylosis].

The case of a 52-year-old patient with systemic amylosis diagnosed upon examination of an osteomedullary biopsy specimen is reported. The most prominent clinical manifestation was a major hemorrhagic syndrome which was recognized as being a result of severe factor Stuart deficiency. The new classification of amyloses based on the latest biochemical and immunological findings is recalled. Current physiopathogenic speculations put emphasis on plasmocyte-macrophage cooperation. Lastly, factor Stuart deficiency, which is as classical as it is uncommon, could originate in the particular affinity of amyloid fibers for this factor and in a "mass effect" (quantity of amyloid substance directly exposed to blood flow), explaining why splenectomy is effective in some instances.

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